Sushma Yalamanchili

Papilloedema due to Chiari I malformation.

The Chiari I malformation is a congenital abnormality characterised by downward displacement of the cerebellar tonsils through the foramen magnum into the cervical spine. It presents clinically most often in young adult women. Known ocular manifestations linked to Chiari I consist primarily of oculomotor paresis with cranial nerve VI palsy and convergence/divergence abnormalities. Papilloedema is a rare manifestation of Chiari I with a clinical presentation often similar to that of idiopathic intracranial hypertension. To highlight this unusual complication, the authors report a 64-year-old female who developed papilloedema as the only presenting neurological symptom resulting from a Chiari I malformation.

Nonorganic Visual Loss in Children

NOVL in children is a relatively common problem for the comprehensive ophthalmologist. Although the etiology and pathophysiology of NOVL are variable, rare cases are because of physical or sexual abuse. Clinical tests can make the diagnosis of NOVL, and nonorganic is our preferred terminology for NOVL. Clinicians should be careful to establish a normal eye examination and prove that the vision is better than claimed before labeling a patient with NOVL. Rarely, the clinical examination is insufficient to establish the diagnosis of NOVL and electrophysiology may be helpful. Reassurance is generally all that is necessary for most children with NOVL, but some patients might benefit from formal psychiatric evaluation. The possibility of nonorganic overlay should also be considered in children with NOVL, and the combination of organic and NOVL is relatively common.

Optic Neuropathy Due to Chronic Lymphocytic Leukemia Proven With Optic Nerve Sheath Biopsy.

Central nervous system involvement from chronic lymphocytic leukemia (CLL) occurs infrequently, and manifestations include cognitive and cerebellar dysfunction and cranial nerve palsies. We report a 45-year-old man with CLL believed to be in clinical remission, who presented with vision loss and bilateral optic disc edema. His optic neuropathy due to CLL was proven by optic nerve sheath biopsy, and he experienced visual recovery after treatment with ibrutinib and intrathecal methotrexate.

Leber Hereditary Optic Neuropathy Mimicking Thyroid-related Optic Neuropathy

this, we carefully reviewed the fluorescein angiogram and found in the early phase of the study that there was good choroidal filling (Fig. 1). In the Photo Essay, Figure 2A shows a later stage of the angiogram when filling of the choroid was complete, yet there was no perfusion of the retinal vessels. Furthermore, the appearance of the vascular occlusion in Figure 2A is actually more suggestive of an occlusive vasculitis than a CRAO caused by emboli, adding further support for the diagnosis of Behçet disease (BD). Second, regarding the issue of diagnostic criteria for BD, our patient admitted to recurrent oral ulcers more than 5 times per year. Finally, we agree that CRAO and recurrent papillitis are not listed as minor features in the diagnostic criteria of BD (2), but “vasculitis” and “central nervous system symptoms” are included. If we accept that the vascular occlusion observed in each eye of our patient was caused by vasculitis and that the optic nerve is part of the central nervous system, then we have 2 minor features of BD. While recurrent oral ulcers are a major feature, it is noteworthy that diagnostic criteria for BD vary and have been revised many times. The International Study Group for BD proposed that the diagnosis be considered when recurrent oral ulcers plus 2 other features are present, in the absence of other clinical explanations (3). In our patient, we performed a wide range of ancillary tests to exclude other disorders, which can cause an obliterative vasculitis. Most importantly, we wish to remind clinicians that CRAO and recurrent papillitis can be caused by a vasculitis, such as BD. Prompt treatment can be helpful to prevent further loss of visual function.

Neuro-Ophthalmology Annual Review.

AbstractThe aim of this study was to update the practicing ophthalmologist on the English-language neuro-ophthalmology literature from the prior year. This study is a review of English-language literature from August 1, 2012, to August 1, 2013. The authors searched PubMed articles published from August 1, 2012, to August 1, 2013, limited to English-language publications including original articles, review articles, and case reports and excluding letters to the editor, unpublished work, and abstracts. We researched the following topics: pupillary abnormalities, eye movement dysfunction, neuromuscular diseases, optic neuropathies, optic neuritis and demyelinating disease including multiple sclerosis, lesions of the chiasm and posterior primary visual pathways, elevated intracranial pressure, tumors and aneurysms affecting the visual pathways, vascular diseases, higher visual functions, and neuroimaging advances. We intend to share clinically relevant literature of the past year with the practicing ophthalmologist. We aimed to highlight remarkable and interesting literature rather than exhaustively including all new neuro-ophthalmological publications of the year. We reviewed literature in the past year with a focus on relevance and novelty. This review updates the comprehensive ophthalmologist on neuro-ophthalmic topics.

Cat scratch neuroretinitis: the role of acute and convalescent titers for diagnosis.

Cat scratch neuroretinitis (CSN) is a clinical diagnosis supported by serological testing. We present 2 cases of CSN in which initial acute titers were negative or equivocal for Bartonella henselae while convalescent titers were shown to be positive. We report these cases to emphasize that a single acute negative titer is insufficient to exclude the diagnosis of CSN and that convalescent titers should be obtained in patients for whom there is a high clinical suspicion of the disease.

Neuro-ophthalmologic features of chordoid glioma

Chordoid glioma is a rare intracranial tumor typically arising in the third ventricle, particularly along the anterior aspect of the hypothalamic wall. We describe the clinical, neuroimaging, and pathologic factors of this neoplasm in a patient presenting with a chiasmal syndrome.

Fourth nerve palsy

What is fourth nerve palsy? There are 12 pairs of nerves (cranial nerves) that control most of the functions of the head and neck. These functions include eye movements, swallowing, facial sensation, and other facial movements. One of those nerves is the fourth cranial nerve. The fourth cranial nerve controls a specific eye movement. If this nerve is not functioning properly on one or both sides of the head, it is called a “palsy.” You may experience double vision and the need to tilt your head. A fourth nerve palsy usually only affects one eye, but it can affect both eyes.

Orbital apex syndrome

A 70-year-old woman presented with a one-month history of progressive, painful loss of vision in the right eye (OD), a droopy right upper eyelid, and horizontal binocular diplopia. Past medical history was significant for hypertension, diabetes mellitus type II, and breast cancer (status post-mastectomy and -chemotherapy). Review of systems was significant for nausea, vomiting, and malaise. On examination, visual acuity was light perception OD and 20/25 in the left eye (OS). The right pupil measured 5 mm in the dark and was non-reactive to light or accommodation with a relative afferent pupillary defect (RAPD) OD (by reverse testing). The left pupil measured 4 mm in the dark and 3 mm in the light with no light-near dissociation. Ocular motility revealed complete ophthalmoplegia OD but full extraocular movements OS (Fig. 13.1). Anterior segment examination and intraocular pressure measurements were normal in both eyes (OU). External exam showed complete ptosis OD. Hertel exophthalmometry showed 3mm of proptosis OD (23mm OD compared with 20mm OS). Dilated fundus exam revealed diffuse optic disc pallor OD and a normal fundus OS. Magnetic resonance imaging (MRI) of the brain and orbits with and without contrast showed a dural-based extra-axial lesion located within the sella and extending into the right-sided cavernous sinus, superior orbital fissure, planum sphenoidale, and infundibulum (Fig. 13.2). Trans-sphenoidal biopsy revealed diffuse large B-cell lymphoma. The patient underwent staging and was treated with systemic chemotherapy and radiotherapy.

Non-arteritic anterior ischemic optic neuropathy (NAION)

A 65-year-old male presented with acute, painless vision loss in the lower portion of his left eye (OS) for one day. Past medical history was significant for hypertension, hyperlipidemia, and coronary artery disease with previous coronary artery stenting. Significant medications included aspirin and clopidogrel (Plavix). He smoked one pack of cigarettes per day for many years. He denied jaw claudication, scalp pain, headache, fever, chills, or malaise and had no history of obstructive sleep apnea. He was not using any phosphodiesterase inhibitors (erectile dysfunction medications). External examination was negative for temporal nodularity or scalp tenderness. Visual acuity was 20/20 in the right eye (OD) and 20/400 OS. The pupils measured 4 mm in the dark and 2 mm in the light in both eyes (OU), but there was a relative afferent pupillary defect (RAPD) OS. The remainder of the anterior segment examination was within normal limits. Automated visual field testing (Humphrey 24-2) was normal OD but showed a dense inferior arcuate field defect OS (Fig. 6.1). Dilated fundus examination OD was normal and showed a cup-to-disc ratio of 0.1 (Fig. 6.2). There was superior (sectoral) disc edema OS (Fig. 6.3) that resolved over time to residual sectoral optic atrophy OS. Optical coherence tomography (OCT) of the optic nerve OS showed a thinned superior nerve fiber layer (Fig. 6.4) six months later. Laboratory workup at the time of